Hemophilia and Thrombosis

The Hemophilia and Thrombosis Program at Children's and UT Southwestern, led by Dr. George Buchanan and associate director Dr. Janna Journeycake, remains a national leader in patient care, education, clinical research and advocacy. It is one of the 130 comprehensive hemophilia programs in the nation, which is funded by the Centers for Disease Control and Maternal Child Health Bureau. Drs. Shelley Crary and Cindy Neunert also serve as faculty in the Program. We manage more than 175 children with bleeding disorders and 150 with thrombophilia/thrombosis. We specialize in the diagnosis and management of children and adolescents with the following conditions:

• Factor VIII and Factor IX deficiency (hemophilia)
• von Willebrand disease
• Factor XI deficiency
• Hypofibrinogenemia
• Factor XIII deficiency
• Qualitative platelet disorders
• Prothrombotic conditions ( i.e., Factor V Leiden, Prothrombin gene mutation, Protein C and S deficiency, antithrombin deficiency, elevated lipoprotein a, antiphospholipid antibodies and lupus anticoagulant)
• Thromboembolic disease, including deep vein thrombosis, pulmonary embolism, catheter-related thrombosis, arterial ischemic stroke and perinatal stroke

Program Highlights

• Comprehensive hemophilia treatment team consists of physicians, an experienced advanced practice nurse, nurse clinician, physical therapist and social worker devoted to the specialized care of children with bleeding and clotting disorders.
• We have leadership in the Transfusion Medicine Hemostasis Network of the National Heart, Lung and Blood Instisute (NHLBI). Dr. Journeycake is the co-chair of the pediatric subcommittee
• Our physical therapist, Alice Anderson, was recognized by the National Hemophilia Foundation (NHF) in 2003 for her outstanding efforts in the treatment and care of children with bleeding disorders.
• Kim Spencer, MSN, CPNP has been an active participant in the National Hemophilia Foundation, including service as co-chair of the Nurse's Working Group and on the planning committees for the Annual Meeting and the National Prevention Campaign.
• Community outreach:
• Nurse coordinators' school visits and lectures to students and staff.
• Education to community physicians and families with inherited thrombophilia about diagnosis, screening and prevention of thrombosis.
• Active participation and support of NHF and the local chapter, TexCen.
• Our social worker, Amanda Cabrera, is on the National Hispanic Patient Advisory Council.
• Close affiliation with UT Southwestern's diagnostic hemostasis laboratory and the adult component of UT Southwestern's hemophilia program.
• Outpatient management of anticoagulation therapy.
• Development of multidisciplinary guidelines for treatment of acute stroke and participation in a comprehensive long-term follow-up clinic for children with stroke.
• In July 2008, 25 Center for Cancer and Blood Disorders patients attended Camp Ailihpomeh, the week-long summer camp for children with hemophilia. In addition, members of our team have served as camp director or co-director for the last four years.

Current and Future Research

• Participation in an NIH-sponsored study using Rituximab for the treatment of inhibitors in patients with hemophilia A.
• Enrollment of 161 patients since May 1999 in the Universal Data Collection study sponsored by the CDC.
• Research on the management of patients with hemophilia and von Willebrand's disease undergoing minor surgical procedures ( Neunert CE, Miller KL, Journeycake JM, and Buchanan GR. Implantable central venous access device procedures in hemophilia patients without an inhibitor: systematic review of the literature and institutional experience. Haemophilia. 2008; 14: 260-270).
• Continued enrollment of patients on the Children's Medical Center Thrombosis Registry and International Pediatric Stroke Registry. We are the major enrolling center in North America in the latter study.
• Participation in the von Willebrand Disease International Prophylaxis Network
• Participation in the International Immune Tolerance Therapy Study.
• Study of catheter complications and their relationship to central venous catheter-related thrombosis in children with cancer and hemophilia (Journeycake and Buchanan Catheter-related deep venous thrombosis and other catheter complications in children with cancer. J Clin Oncol. 2006; 24: 4575-80).
• Ongoing study of the thromboembolic complications related to splenectomy (Troendle SB, Adix L, Crary SE, Buchanan GR. Laboratory Markers of Thrombosis Risk in Children with Hereditary Spherocytosis. Pediatr Blood Cancer 2007; 49: 781-785 and Crary SE, Troendle S, Torres R, Ahmad N, Buchanan GB. Prospective Assessment of Laboratory Markers of Cardiovascular Risk Following Splenectomy in Hereditary Spherocytosis, oral paper presentation at the ASPHO annual meeting, Cincinnati, OH, May 2008)

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